Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Caroli's disease is defined as a abnormal dilatation of the intra-hepatica bile ducts: Its incidence is extremely low (1 in 1,000,000 population) and in most of the cases the whole liver is interested and liver transplantation is the treatment of choice. In case of dilatation limited to the left or right lobe, liver resection can be performed. For many year the standard approach for liver resection has been a formal laparotomy by means of a large incision of abdomen that is characterized by significant post-operatie morbidity. More recently, minimally invasive, laparoscopic approach has been proposed as possible surgical technique for liver resection both for benign and malignant diseases. The main benefits of the minimally invasive approach is represented by a significant reduction of the surgical trauma that allows a faster recovery a less post-operative complications. This video shows a case of Caroli s disease occured in a 58 years old male admitted at the gastroenterology department for sudden onset of abdominal pain associated with fever (> 38 C degrees), nausea and shivering. Abdominal ultrasound demonstrated a significant dilatation of intra-hepatic left sited bile ducts with no evidences of gallbladder or common bile duct stones. Such findings were confirmed abdominal high resolution computer tomography. Laparoscopic left sectoriectomy was planned. Five trocars and 30 degrees optic was used, exploration of the abdominal cavity showed no adhesions or evidences of other diseases. In order to control blood inflow to the liver, vascular clamp was placed on the hepatic pedicle (Pringle s manouvre), Parenchymal division is carried out with a combined use of 5 mm bipolar forceps and 5 mm ultrasonic dissector. A severely dilated left hepatic duct was isolated and divided using a 45 mm endoscopic vascular stapler. Liver dissection was continued up to isolation of the main left portal branch that was then divided with a further cartridge of 45 mm vascular stapler. At his point the left liver remains attached only by the left hepatic vein: division of the triangular ligament was performed using monopolar hook and the hepatic vein isolated and the divided using vascular stapler. Haemostatis was refined by application of argon beam coagulation and no bleeding was revealed even after removal of the vascular clamp (total Pringle s time 27 minutes). Postoperative course was uneventful, minimal elevation of the liver function tests was recorded in post-operative day 1 but returned to normal at discharged on post-operative day 3.
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PMID:Laparoscopic left liver sectoriectomy of Caroli's disease limited to segment II and III. 1925 71

A 38-year-old man presented with pain in the right upper abdomen combined with elevated C-reactive protein (5.37 mg/dl) and creatinine levels (2.0 mg/dl). The medical history revealed recurrent abdominal pain. Duplex sonography showed large intrahepatic cystic structures, partially combined with a ribbon-shaped flow signal inside, as well as multiple cysts in the kidneys. The following MRI identified these findings as dilatated bile ducts surrounding the portal vein branches. This is known as the central dot sign. Overall, this special constellation is indicative of Caroli's disease, a rare congenital cystic dilatation of the intrahepatic bile ducts. The imaging results substantiated the diagnosis, especially in combination with cystic renal disease and recurrent cholangitis. Depending on the grade of liver involvement the treatment is partial hepatectomy or, as in our case transplantation, which also eliminates the risk of associated cholangiocarcinoma.
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PMID:[Upper abdominal pain with recurrent cholangitis and pyelonephritis]. 2017 81

We present the case of a 65-year-old male patient without any family history of renal or hepatic disease. He had been on maintenance hemodialysis for 4 months because of autosomal recessive polycystic kidney disease. At the start of the hemodialysis session he reported general malaise, abdominal pain, fever and diarrhea occurring in the last two days. Laboratory workup showed neutrophilic leukocytosis and increased serum amylase and C-reactive protein. Abdominal contrast-enhanced CT scan and MRI cholangiography showed hepatic cysts with marked dilatation of the intra- and extrahepatic bile ducts. The patient underwent cholecystectomy with hepaticojejunal Rouxen- Y anastomosis and was discharged with oral ciprofloxacin. Histology confirmed marked cystic dilatation of the bile ducts. Because of persistent episodes of septic fever, administration of ciprofloxacin was continued. After 4 months retrograde endoscopic pancreatography was performed which led to a diagnosis of Caroli's syndrome associated with polycystic kidneys. Given the rarity of the disease and its difficult diagnosis, when patients with polycystic kidneys and liver cysts experience recurrent episodes of septic fever of unknown origin, Caroli's disease should be taken into account and the appropriate tests should be carried out to confirm the diagnosis.
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PMID:[Caroli's syndrome in a patient on hemodialysis: diagnostic and therapeutic difficulties]. 2092 88

Abernethy malformation is a rare anomaly with partial or complete congenital absence of the portal vein and subsequent development of extrahepatic portocaval shunts. We present the case of a 28-year-old woman who was incidentally diagnosed with type II Abernethy malformation and multiple aneurysms during an investigation for nonspecific abdominal pain and fever. The patient had been diagnosed with Caroli's disease at the age of 10 and liver cirrhosis, portal hypertension a few years before. To the best of our knowledge, this is the first case reported with all such congenital anomalies associated together. Ultrasound, computed tomography, including three-dimensional reconstruction, and magnetic resonance imaging were performed which revealed a side-to-side shunt between the extrahepatic portal vein and the inferior vena cava, multiple aneurismal cystic dilation of the spleen artery and left renal artery, and extensive intrahepatic bile duct cysitic dilation with calculus formation. Etiology, clinical significance and management strategies with regard to these abnormalities are discussed.
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PMID:Abernethy malformation with multiple aneurysms: incidentally found in an adult woman with Caroli's disease. 2339 46

Caroli's disease is a rare congenital disorder characterized by cystic dilatation of the large in-trahepatic bile ducts. The most frequent complications due to biliary stasis are cholelithiasis, cholangitis and sepsis as well as an increased risk of cholangiocarcinoma. Patients may have a history of intermittent abdominal pain, pruritus and/or symptoms of cholangitis. It is rarely diagnosed in childhood. A 12-year-old boy with isolated Caroli's disease is described. This child presented at the age of 2 years, with 4 episodes of recurrent bacterial infections. Interestingly he remained asymptomatic for over 10 years, between the second and third episode. During the 4th episode, when he presented with fever and slight abdominal pain, the diagnosis was made on the basis of radiological findings: U/S, CT, MRI and especially with MRCP, in relation with a more typical picture, resembling cholangitis. Since then he has been followed-up systematically for ten years and remains in good clinical condition without further relapses and with unchanged radiological findings. This atypically benign course of Caroli's disease, with intermittent asymptomatic periods, without any treatment, is very rare.
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PMID:Caroli's disease: Description of a case with a benign clinical course. 2471 23

This paper describes a case of Caroli's disease in a female patient aged 32, who complained of nonspecific abdominal pain without cholesthasis or cholangitis. Liver resonance shows segment saccular dilations closely connected to intrahepatic biliary ducts, that differ from the Caroli's syndrome, which is more common and consists of multiple intrahepatic cystic dilatations, associated to congenital hepatic fibrosis. This patient has a congenital anomaly with an uncommon oligosymptomatic form of Caroli's disease that should be included in the differential diagnosis of patients with recurrent abdominal pain.
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PMID:Diffuse Caroli's disease with atypical presentation: a case report. 2471 20

Caroli's disease is a rare congenital condition characterized by segmental non-obstructive fibrocystic dilation of intrahepatic bile ducts. Dilated ducts may be infected and contain stones. This review of the modern literature describes few known cases of the disease, analyses its clinical features and results of treatment. The principal diagnostic methods include visualization by ultrasound study, CT, MRT, retrograde and transhepatic cholangiography. Surgical intervention is the method of choice for the treatment of the disease including resection of liver, placement of hepatico-jejunal anastomoses and transplantation. The choice depends on the extent of the lesion and anticipated complications. The authors' observation of the patient with Caroli's disease in a 35 year-old woman is presented. It was successfully treated by left-hand bisegmentectomy even though the disease was diagnosed 14 years after the first symptoms. It is recommended that the disease, even if a rare one, should be included in the algorithm of differential diagnostics ofrecurrent abdominal pain with manifestations of cholestasis or fever of an unknown origin.
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PMID:[Caroli's disease: diagnostic problems and possibilities of treatment]. 2569 53


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